angiomatoid fibrous histiocytoma

angiomatoid fibrous histiocytoma

An intermediate-grade malignancy surrounded by a dense fibrous pseudocapsule which occurs in younger patients (usually under age 20; rarely, up to age 40).

Clinical findings
Pain, fever, anaemia, weight loss, malaise, anorexia, paraproteinaemia, increased cytokine production.

Prognosis
10% recurrence, rare metastasis.
References in periodicals archive ?
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue neoplasm of intermediate (rarely metastasizing) biologic potential and uncertain differentiation, which predominantly arises superficially in the deep dermis and subcutis of the extremities of children and young adults.
Angiomatoid fibrous histiocytoma is rare, accounting for approximately only 0.
Angiomatoid fibrous histiocytoma does not have a proven relationship with any other specific conditions, although one case has been reported on the knee of a child with human immunodeficiency virus.
Angiomatoid fibrous histiocytoma shows a wide morphologic spectrum, and the only constant finding is of sheets and short fascicles of ovoid, epithelioid, or spindle cells with bland, vesicular nuclei (Figure 1).
Angiomatoid fibrous histiocytoma lacks a specific immunoprofile, so that immunohistochemistry is supportive rather than diagnostic.
Angiomatoid fibrous histiocytoma is associated with the following 3 characteristic translocations" t(2:22)(q33:q12) (forming the EWSR1-CREB1 fusion gene), (43,44) t(12:22)(q13:q12) (forming the EWSR1-ATF1 fusion gene) (7,44-46) and t(12:16)(q13:p11) (resulting in the FUS-ATF1 fusion gene).
Angiomatoid fibrous histiocytoma is a rare neoplasm of intermediate biologic potential most frequently occurring in the superficial extremities of children and young adults.
Intracranial angiomatoid fibrous histiocytoma presenting as recurrent multifocal intraparenchymal hemorrhage.
Angiomatoid fibrous histiocytoma of bone" a calcifying sclerosing variant mimicking osteosarcoma.
Imaging characteristics of angiomatoid fibrous histiocytoma of bone.
Angiomatoid fibrous histiocytoma as a second tumor in a young adult with testicular cancer.
24) The frequencies of these translocations vary between tumors; EWSR1-CREB1 is the most frequent gene fusion in angiomatoid fibrous histiocytoma (compared with EWSR1-ATF1 or FUSATF1), (26) whereas most conventional CCSs display EWSR1ATF1 fusions, with only a subset harboring EWSR1CREB1.